Nandivada Prathima, Potemkin Alexis K, Carlson Sarah J, Chang Melissa I, Cowan Eileen, O'Loughlin Alison A, Gura Kathleen M, Puder Mark
The Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Department of Pharmacy, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
JPEN J Parenter Enteral Nutr. 2015 Nov;39(8):973-6. doi: 10.1177/0148607114545995. Epub 2014 Aug 8.
Elevated serum alkaline phosphatase (ALP) in infants with intestinal failure (IF) can be due to parenteral nutrition-associated liver disease (PNALD) or metabolic bone disease (MBD). The purpose of the study was to determine the utility of serum ALP in the diagnostic criteria for PNALD by measuring tissue-specific levels in infants with IF and PNALD.
A retrospective review of patient data for 15 infants diagnosed with PNALD between December 2012 and August 2013 was performed. PNALD was defined as the presence of 2 consecutive direct bilirubin (DB) levels >2 mg/dL. Fractionated serum alkaline phosphatase was measured in each patient, while the DB was >2 mg/dL. Parathyroid hormone (PTH), vitamin D3, calcium, and phosphate levels were recorded where available.
In 15 infants with PNALD, elevation in total ALP was due to marked elevations in bone-specific ALP. The median liver-specific ALP remained within the normal range. PTH, vitamin D3, calcium, and phosphate levels were within normal limits.
While elevated ALP can reflect biliary stasis, the ALP elevation observed in infants with IF and PNALD is predominantly of bone rather than hepatic origin. An elevated unfractionated ALP in infants with PNALD should therefore raise suspicion of underlying bone disease, rather than being attributed to liver disease alone.
肠衰竭(IF)婴儿血清碱性磷酸酶(ALP)升高可能是由于肠外营养相关肝病(PNALD)或代谢性骨病(MBD)。本研究的目的是通过测量IF和PNALD婴儿的组织特异性水平,确定血清ALP在PNALD诊断标准中的效用。
对2012年12月至2013年8月期间诊断为PNALD的15例婴儿的患者数据进行回顾性分析。PNALD定义为连续2次直接胆红素(DB)水平>2mg/dL。在每位患者DB>2mg/dL时测量血清碱性磷酸酶同工酶。如有可用数据,记录甲状旁腺激素(PTH)、维生素D3、钙和磷水平。
在15例PNALD婴儿中,总ALP升高是由于骨特异性ALP显著升高。肝脏特异性ALP中位数仍在正常范围内。PTH、维生素D3、钙和磷水平均在正常范围内。
虽然ALP升高可反映胆汁淤积,但IF和PNALD婴儿中观察到的ALP升高主要源于骨骼而非肝脏。因此,PNALD婴儿中未分级的ALP升高应引起对潜在骨病的怀疑,而不应仅归因于肝病。